Ingabe ukhona emndenini wakho, ikakhulukazi ingane, onenkinga yama-hormone azalwa nawo? Ungase ujwayelene nezinkinga ezifana nokukhubazeka kokukhula, ukuthomba kwasekuqaleni, kanye nemijikelezo yokuya esikhathini engajwayelekile kwabesifazane. Isimo esingavamile sofuzo esingabangela lokhu sibizwa ngokuthi 'i-Congenital Adrenal Hyperplasia'. Sizosibiza ngokuthi i-CAH ngamafuphi. Namuhla, sizokhuluma kafushane ngalokhu kanye nokwelashwa okusha okufikile ngakho.
Kalula nje, iyini i-CAH?
I-CAH yisimo sofuzo esivimbela izindlala ze-adrenal, izindlala ezimbili ezincane ezitholakala ngaphezu kwezinso, ekukhiqizeni i-cortisol, i-hormone ebalulekile empilweni yethu.
Cabanga ngemizimba yethu njengefektri. Uma omunye wemikhiqizo (i-cortisol) kule fektri uphelelwa amandla, isikhungo sokulawula ebuchosheni (i-pituitary gland) siyaphazamiseka bese sithumela izimpawu (i-ACTH) ezindlaleni ze-adrenal ukuze zisebenze kanzima. Ngenxa yalokhu kukhuthaza ngokweqile, yize ukukhiqizwa kwe-cortisol kungandi, kunalokho kuqala ukukhiqiza ama-hormone amaningi wesilisa abizwa ngokuthi 'ama-androgens' .
Lokhu kungalingani kwama-hormone kungabangela izinkinga eziningi:
- Izingane zesifazane: Lapho zizalwa, izitho zangasese zangaphandle zingase zibukeke njengezomntwana wesilisa.
- Kubo bobabili abantu abathintekayo: izinkinga ezifana nokukhubazeka kokukhula, ukuthomba kwasekuqaleni, kanye nokungabi nabantwana zingase zenzeke kamuva ekuphileni.
Ngaphezu kwalokho, ezinye iziguli ze-CAH zingase zibe nokukhiqizwa okunciphile kwenye i-hormone ebalulekile ebizwa ngokuthi i-aldosterone. Ngaphandle kwale hormone, i-CAH ingaba nzima kakhulu, okuholela ekushaqekeni, ekukhomeni, noma ngisho nasekufeni.
Ukwelashwa kweminyaka engu-70 kanye nethemba elisha
Kwaphela amashumi eminyaka, ukwelashwa okuwukuphela kwe-CAH kwakuwukufaka esikhundleni samazinga aphansi e-cortisol emzimbeni ngemithamo ephezulu yama-steroid , njenge-hydrocortisone. Kodwa-ke, ukuthatha le mithamo ephezulu yama-steroids isikhathi eside kungabangela imiphumela emibi eminingana. Okuvame kakhulu kulokhu ukukhuluphala, umfutho wegazi ophakeme, amathambo abuthakathaka, kanye nokukhula okungavamile ezinganeni.
Kodwa manje kunezindaba ezinhle! Ngemva kweminyaka engama-70, i-FDA ivumile umuthi wokuqala nowodwa owenzelwe ngqo i-CAH, i-crinecerfont (igama lomkhiqizo: i-Crenessity).
Lo muthi omusha (i-Crinecerfont) usebenza kanjani?
Lena yinto ebaluleke kakhulu. Ngokungafani nokwelashwa okudala, lo muthi omusha usebenza ngendlela ehluke ngokuphelele.
I-'Crinesurfont' ayifaki ama-hormone avela ngaphandle. Esikhundleni salokho, lo muthi uya ngqo esikhungweni sokulawula sobuchopho (i-pituitary gland) futhi uvimbele ukukhishwa kwesignali engafuneki (ACTH) evuselela izindlala ze-adrenal.
Kalula nje, lokhu kufana nokuvala 'iswishi' yesistimu engadingeki. Lokhu kulawula ukukhiqizwa komzimba kwama-hormone 'androgen' angadingekile.
Inzuzo enkulu yalokhu ukuthi njengoba amazinga e-androgen elawulwa, umthamo wama-steroid adingekayo ukugcina ibhalansi yama-hormone ungancishiswa kakhulu . Lokhu kusho ukuthi imiphumela emibi ebangelwa ama-steroids nayo ingancishiswa kakhulu.
| Isici | Ukwelashwa okudala (imithamo ephezulu yama-steroid) | Ukwelashwa okusha (i-Crinecerfont + imithamo ephansi ye-steroid) |
|---|---|---|
| Ukusebenza kahle | Ukuhlinzeka nge-cortisol hormone encishisiwe ngaphandle. | Ukuvimba izimpawu ezingafuneki (ACTH) ebuchosheni nokulawula ukukhiqizwa kwe-androgen. |
| Inhloso eyinhloko | Kudingeka imithamo ephezulu ukulawula amazinga ama-hormone. | Ukwehlisa amazinga e-androgen nokunciphisa umthamo wama-steroid okudingekayo. |
| Okuqhamuka uma udla imishanguzo | Izinkinga zesikhathi eside ezifana nokukhuluphala, ukukhula okulinganiselwe, kanye namathambo abuthakathaka. | Kungenzeka kube nemiphumela emibi ehlobene nama-steroid encishisiwe. Kubikwe imiphumela emibi emincane (ikhanda elibuhlungu, ukukhathala). |
Ubani lo muthi ofaneleka kakhulu?
I-'Crinesurfont' okwamanje ivunyelwe iziguli ze-CAH ezingaphezu kweminyaka engu-4 ubudala . Akuyona indawo yama-steroid, kodwa iwukungezwa kwama-steroids.Ukwelashwa okungeziwe.
- Ezinganeni: Njengoba izingane zisakhula, imithamo ephezulu yama-steroid ingaba nomthelela omkhulu ekukhuleni kwazo. Ngakho-ke, lo muthi, onganciphisa umthamo wama-steroids, ungaba usizo kakhulu kuzo. Lokhu kubaluleke kakhulu ngesikhathi sobusha, lapho kunzima ukulawula ama-hormone.
- Kwabantu abadala: Lokhu kwelashwa kungaphinde kunikeze impumuzo kubantu abadala abahlushwa yimiphumela emibi ngokuthatha ama-steroid isikhathi eside, noma abanobunzima bokugcina amazinga ama-hormone azinzile.
Funda ngemiphumela emibi kanye nokuphepha
Ezivivinyweni zemitholampilo, kubikwe imiphumela emibi emincane yesikhashana kuphela ngalo muthi.
- Kwabantu abadala: ukukhathala, ikhanda elibuhlungu, isiyezi, ubuhlungu bamalunga.
- Ezinganeni: ikhanda elibuhlungu, isisu esibuhlungu, ukukhathala, ikhala elicijile.
Okubalulekile ukuthi iningi lalezi zimpawu lalilincane, kanti inani leziguli okwadingeka ziyeke ukuthatha imithi laliphansi kakhulu.
Isexwayiso Esikhethekile: Qaphela Inkinga Yezinso!
Uma uthatha lo muthi, kubaluleke kakhulu ukuqaphela isimo esibucayi esibizwa ngokuthi 'i-adrenal crisis'. Kalula nje, lesi simo singavela uma umzimba ungatholi inani elidingekayo le-'cortisol' ngesikhathi sokucindezeleka okukhulu (isib. ukutheleleka okukhulu, ukuhlinzwa, ingozi).
Ngakho-ke, khuluma nodokotela wakho futhi uqiniseke ukuthi uyazi kahle ukuthi yiliphi idosi le-steroid elengeziwe okufanele ulithathe esimweni esiphuthumayo ("idosi yokucindezeleka") . Futhi, qaphela izimpawu 'zenkinga ye-adrenal' (isb., ukuhlanza ngokweqile, uhudo, isiyezi, ukulahlekelwa ukwazi) futhi ukhumbule ukuya esibhedlela noma ekamelweni lezimo eziphuthumayo (ETU) ngokushesha uma kwenzeka into enjalo.
Ngingazi kanjani ukuthi lo muthi ulungele mina/ingane yami?
Lesi sinqumo kufanele senziwe ngokubonisana nodokotela wakho, ikakhulukazi isazi se-endocrinologist . Udokotela wakho uzocabangela izici eziningi, okuhlanganisa imiphumela yokuhlolwa kwegazi lakho, izinga lokukhula, isisindo, umfutho wegazi, kanye namazinga ama-hormone amanje, ukuze anqume ukuthi lo muthi ukufanele yini.
Lokhu akulona ikhambi le-CAH, kodwa kunethemba elikhulu lokuthi kuzosiza ekuthuthukiseni ikhwalithi yokuphila kweziguli, kunciphise imiphumela emibi ye-steroid, futhi kwenze ukuphathwa kwama-hormone kube lula.
Umlayezo Wokuya Nawe Ekhaya
- I-CAH isho ukuthiIsimo sofuzo lapho izindlala ze-adrenal zikhiqiza khona i-cortisol encane kanye nama-hormone e-androgen amaningi.
- I-Crinecerfont iyindlela entsha yokwelapha eyenzelwe ngqo i-CAH, eyokuqala yohlobo lwayo eminyakeni engu-70.
- Lona akusiwo umuthi othatha indawo yama-steroid. Ngumuthi onikezwa ngaphezu kwama-steroids futhi osiza ekunciphiseni umthamo wama-steroids adingekayo.
- Inzuzo eyinhloko yikhono lokunciphisa imiphumela emibi yesikhathi eside yama-steroid.
- Qiniseka ukuthi ubuza udokotela wakho ngesimo esiphuthumayo esibizwa ngokuthi 'i-adrenal crisis' kanye "nomthamo wokucindezeleka" okufanele uwuthathe phakathi naleso sikhathi.
- Kubalulekile ukubonisana nodokotela ochwepheshe bezifo zama-hormone ukuze kutholakale ukuthi lokhu kwelashwa kulungile yini kuwe noma enganeni yakho.
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